Damn Interesting

The Jumping Frenchman of Maine Disorder

In a nutshell, evolution describes the fact that in a given set of organisms, those which manage to survive are the same ones that do most of the breeding. It naturally follows that the traits of the survivors become the most prevalent in the species. Perhaps one of the first traits to be promoted by evolution was the survival instinct itself, since the two reinforce one another so elegantly.

As part of the survival instinct, most animals (including humans) react to sudden, unexpected stimuli with a startle reaction, which includes reflexive movement away from the stimulus and a contraction of the muscles in the limbs. It also causes changes in blood pressure, respiration, and breathing. In a normal individual, the muscular reaction subsides within a couple seconds if no real threat is detected, but for a sufferer of the Jumping Frenchman of Maine Disorder, an unexpected stimulus results in a somewhat different experience.

An individual with this disorder has a genetic mutation that prevents “exciting” signals in the nervous system from being regulated, which causes a number of bizarre irregularities in their startle response. Most notably, an event which might startle a normal person will result in an extended, grossly exaggerated response from a “jumper,” including crying out, flailing limbs, twitching, and sometimes convulsions. Because a jumper is almost immediately susceptible to another jump soon after an episode ends, there have been reports that sufferers are sometimes teased mercilessly by people who find the reaction amusing, and trigger it repeatedly.

Another curious abnormality caused by this disorder is a sufferer’s automatic reflex to obey any order that is delivered suddenly. For example, if one uses a sharp, quick voice to order a jumper to throw the object in their hands, they will throw it without hesitation; if they are similarly told to strike a person, they will strike that person, even if it is a loved one. Very often, if an individual with this disorder hears a phrase that is unfamiliar or spoken in a loud voice, they will uncontrollably repeat that phrase back, a phenomenon known as echolalia.

The Jumping Frenchman of Maine Disorder was first described by G. M. Beard in 1878 after observing the effect in French-Canadian lumberjacks in the Moosehead Lake area of Maine:

“One of the jumpers while sitting in his chair with a knife in his hand was told to throw it, and he threw it quickly, so that it struck in a beam opposite; at the same time he repeated the order to throw it…. When the commands are uttered in a quick loud voice the jumper repeats the order. When told to strike, he strikes, when told to throw it, he throws it, whatever he has in his hands…. all of these phenomena were indeed but parts of the general condition known as, jumping. It was not necessary that the sound should come from a human being: any sudden or unexpected noise, as the explosion of a gun or pistol, the falling of a window, or the slamming of the door, provided it be unexpected and loud enough, would cause the jumpers to exhibit some one or all of these phenomena….” (Beard, 1880a, pp. 487-490)

This highly unusual disorder has also been observed in a few other parts of the world, including Siberia and Malaysia. Jumping Frenchman is a form of Hyperexplexia, a more generic term descriptive of any exaggerated startle response. Jumping Frenchman bears similarities to Stiff Baby Syndrome, a condition where a person has an exaggerated startle response from the time they are born, and a tendency as a young child to occasionally stiffen their whole body in response to surprise stimuli. This causes the child to fall to the floor like a log when surprised, not unlike the genetic oddity of “fainting goats” that involuntarily stiffen their whole bodies when startled. Jumping Frenchman and Stiff Baby are so similar that they are thought by many to be the same disorder. When Beard first described Jumping Frenchman in 1878, Dr. Gilles de la Tourette attempted to have it classified as part of the syndrome which bore his name, but this notion was rejected due to the distinct differences between Tourette Syndrome and Jumping Frenchman.

Most evidence seems to indicate that hyperexplexia is caused by a genetic mutation which prevents neurons from receiving the amino acid glycine. Glycine in an inhibitory neurotransmitter common in the spinal cord, and it prevents the central nervous system from overreacting to stimuli. The poison strychnine also inhibits glycine reception, and the symptoms it causes are very similar to hyperexplexia.

In the case of the original test subjects in Maine, it appears that the problem mutation might have been caused by inbreeding, however some researchers believe that the disorder is psychological rather than neurological. The debate is still open. Either way, it is always fascinating to see what happens when the human brain experiences a short circuit. Indeed, truth is sometimes stranger than fiction… sometimes to a startling degree.

Did the Corps go PC? If that change is legit, that would be Damn Interesting.

megzee

Posted 12 March 2006 at 02:59 am

Enter your reply text here.

megzee

Posted 12 March 2006 at 03:06 am

Oops. My cat stepped on the keyboard…anyway…I think I knew someone with this disorder. I’ve worked with special-needs kids in the past. As for making a super soldier, there’s no way. The startle response would completely take over their ability to be good at what they do. Imagine…suprise gunfire, and being unable to react appropriately, taking too much time to find cover. They’d be dead men. And if someone told them to fire their gun, one of their comrades would be hurt or killed.
I do agree with Chastian Troy, though. It does sound like a drill instructor (I was Army, though). Been there done that.

Stuart

Posted 13 March 2006 at 02:32 am

Berkana said: “Who knew inbreeding could be such an endless source of entertaining disorders?”

I remember seeing an article on a mountain village in India were the interbreeding has been going on for so long that most of the village is deaf (about 80% if I remember correctly) so everyone communicates with sign language. No spoken words are used. There was a brilliant explanation from a Delhi based doctor, “The problem is genetic: they keep marrying each other!”

God of Biscuits

Posted 13 March 2006 at 10:01 am

I myself am the direct product of inbreeding and I would like you all to take extra care with your words, as some of them are offensive and dehumanizing to me and others like me.

rp2

Posted 14 March 2006 at 10:38 am

hehe.. you’re funny

Fredrated

Posted 18 March 2006 at 02:02 pm

“evolution describes the fact that in a given set of organisms, those which manage to survive are the same ones that do most of the breeding.”

Do we know how much of the breeding is done by those that don’t survive?

The_Smurf_Strangler

Posted 23 March 2006 at 09:07 am

It would be mean, but there should be a whole section of the wonders of inbreeding….kind of a online sideshow……ya

Nytemare3701

Posted 24 March 2006 at 01:31 am

Oddly enough, inbreeding was made illegal in the US and now US citizens have this EEEEEWWWW complex over it.

on another note, If you stick said super-soldiers in a bunker withguns pointed towards the enemy, you could repeatedly make them spook each other with gunfire, and you would have a great frontline defense!
At that point you should just use computer controlled turrets though…

I don’t know where you got your ideas for paragraphs 3 & 4. “Very “creative.” In-breeding? Genetic mutation? Don’t you reference what you declaim as “fact?”

OK. The fainting goat video was funny.

Those men in Maine were miners working with nerve-shattering explosives.

The stuff about doing things as told is a little weird. Interesting that Dr. Tourette wanted to investigate.

These “Frenchmen” were not in-bred. They were Quebeqouis Canadians.

I just googled hyperexplexia. Evidently, so did you. Stiff Baby Syndrome is more rare than Stiff Man/Jumping Frenchmen/Moersch-Woltman Syndrome. We’re not a rare disease. We’re an orphan disease. A syndrome. A conundrum. Looking to be adopted.

speaking of incompetence, did you even read the linked websites? look at this, copied from the third link, which points out that “stiff man” is a separate condition from jumping frenchman:

“It is important to distinguish the stiff baby syndrome from other neurological diseases manifest by an increase in muscle tone. First, this syndrome must not be confused with the stiff man syndrome described bv Gordon et al in 1967.[8] The stiff man syndrome is not hereditary and appears at the age of 40 to 60; evolution is slow and progressive. It is distinguished by a permanent contracture, an intense dysphagia, the electromyogram shows a permanent electric activity, even during rest, and it is magnified by the slightest tactile or nociceptive stimulus.[9] The Isaacs-Mertens syndrome, usually occuring around the age of 30, and for which neonatal forms have been reported, presents a mostly distal hypertonia, accompanied by fasciculations; the eclectromyogram shows a permanent activity on resting as well as numerous discharges. The syndrome described by Stevens in 1965 under the name ‘Jumping Frenchman of Maine’ is different, as violent starts, produced by the slightest stimulus, are associated with an echolalia and an echopraxia.[10] Gilles de la Tourette’s syndrome chiefly presents spasmodic twitchings and startling reactions. Several other conditions can produce abnormalities in tonicity: among them are the encephalomyelites, Creutzfeldt-Jakob disease, metoclopramide or strychnine poisoning, and myoclonic epilepsy.”

you seem to be illustrating the thrust of the incompetence piece quite nicely… speaking with authority on a subject you obviously lack knowledge in.

maux

Posted 28 March 2006 at 11:05 am

Oh, please. The dood who wrote this article used info from a 19th century document.

I read the non-enlightening links. There’s been no genetic link found. Nor other “frenchmen” still chopping trees in Maine (not mining, my bad.)

Come out from your anonymous mask. Your ability to cut and paste is quite competent. Will check back later for more insults.

maux

Posted 12 April 2006 at 08:46 am

Dearest idiots,

Do not tell me to reference wikipedia. It only satisfies your total ignorance.

You are all idiots. And incompetent. Do you have on-line access to medline? Not. Stop your misleading “reporting.”

Thanks for kindly NOT REPLYING, Assholes.

bub-bye.

Alan Bellows

Posted 12 April 2006 at 10:28 am

maux said: “You are all idiots. And incompetent. Do you have on-line access to medline? Not. Stop your misleading “reporting.””

Dear maux,

Citations are more convincing than insults. If our facts are off, we’ll happily correct them… but we do need a source more concrete than your unsupported statements (particularly considering your credibility-killing over-the-top name-calling).

Hugs and kisses.

sleepwalker

Posted 18 April 2006 at 01:22 am

our housemaid got the same dis-order.

if you shout a snappy command, she would repeat it… and often more than once. we always thought she got nervous problem.

if i will hold her hand, look her hard in the eyes seriously… and suddenly jump or dance while yelling, she would do the same. after that, it will be unstoppable reactions.

if i will grab the hair of the person next to her, she would do it also even after i stop doing it till we restrain her.

The “Jumping Frenchmen of Maine” were described by George Beard in 1878. They had an excessive startle response, sometimes with echolalia, echopraxia, or forced obedience. In 1885, Gilles de la Tourette concluded that “jumping” was similar to the syndrome that now bears his name. Direct observations of jumpers have been scarce. We studied eight jumpers from the Because region of Quebec. In our opinion, this phenomenon is not a neurologic disease, but can be explained in psychological terms as operant conditioned behavior. Our cases were related to specific conditions in lumber camps in the 19th and the beginning of the 20th century.

The startle response is a universal and phylogenetically ancient reflex. Pathological exaggerations and modifications of startle underlie the symptomatology of a surprisingly diverse range of neuropsychiatric disorders, a fact that seems to have gone largely unappreciated. We review the available literature on the physiology of the normal human startle response and examine the neuropsychiatric conditions characterized by pathological startle. Startle epilepsy and primary hyperekplexia are considered as neurological disorders involving abnormal startle. Patients with hyperstartle and exotic culture-bound syndromes characterized by excessive startle are considered by the authors to represent extreme variants of the normal startle response. Post-traumatic stress disorder, drug and alcohol withdrawal states and schizophrenia all have abnormal startle as a clinical feature secondary to increased arousal and presumed disturbance of central neurotransmitter systems. The neurophysiological mechanisms by which abnormalities of the startle response may occur are discussed and a system of classification of neuropsychiatric hyperstartles is suggested.

talking about Saint-Hilaire ; it’s a little town in the French Alps. Once a year in september they have the biggest and best free flight airshow in the World. 100,000 people come and watch pilots jump of this humongus cliff with paragliders and hanggliders doing acro or they dress up.

All the bickering is interesting but the article by Saint-Hilaire MH, Saint-Hilaire JM, and Granger L. is correct. The problem they had is that they could not name the source of stimulus to cause operant conditioning. That source was visual Subliminal Distraction. That is the name, outside the United States, for stimulus created by a “conflict of the physiology of sight.”

Designers accidentally discovered the problem when knowledge workers using the first prototypes of close-spaced office workstations began to have mental breaks. The Cubicle solved that problem by 1968.

Jumping Frenchmen of Maine disappeared when modern logging equipment replaced bunkhouses of lumberjacks in remote lumber camps. Latah, the startle matching behavior of Malaysia, happens when entire villages live in longhouses. Around the world traditional or ethnic housing consists of small single-room living arrangements. These situations allow exposure to Subliminal Distraction if one member of the group attempts some activity requiring full mental investment while other group members move in the person’s Subliminal Peripheral Vision.

Mental breaks from this exposure happen to users of Qi Gong and Kundalini Yoga. They have happened aboard US Navy submarines-Screaming Seaman, on polar expeditions – Belgian Polar Expedition of 1898, and on Russian space missions -Soyuz-21.

The “conflict of physiology” is that although you can ignore the movement that triggers a peripheral vision reflex, basic facts of the physiology of sight mean you can’s stop-seeing anything in any portion of your vision field. The subliminal operation of the system prevents your being aware your subconscious is being bombarded by attempts to force a vision reflex.

The Jumping Diseases, Startle Matching Behaviors, are so strange they cannot be argued to be something else. They provide a unique understanding of the potential for exposure to Subliminal Distraction.

Culture Bound Syndromes, mental events that happen around the world, also happen when Subliminal Distraction is present. They evidence a variety of psychiatric symptoms the phenomenon can produce.

CauselessEffect

Posted 14 September 2006 at 06:27 pm

Why does every forum have someone who’s sole purpose is to attack others? Resorting to foul language is the lowest form of insult and is no way to debate your stance. The writers here at Damn Interesting do a great job and they do it for our pleasure. I just don’t understand how someone can get so angry over something so silly.

denki

Posted 12 October 2006 at 10:10 pm

When I become a crazy megalomaniac (as opposed to a sane megalomaniac) I am totally going to have these guys as my bodyguards/jesters.

psyOtic

Posted 13 October 2006 at 12:21 am

God of Biscuits said: “I myself am the direct product of inbreeding and I would like you all to take extra care with your words, as some of them are offensive and dehumanizing to me and others like me.”

a disturbing point to look at with inbreeding we look at it in current times as disgusting and absurd how ever we all at some point in our family tree are the result of inbreeding… (of course thats only if you believe in evolution.)

as for genetic deformities they are the down side of evolution some people may develop a unique ability or be born with a stronger sense of some type note an article done a while ago about people being able to see extra colours https://www.damninteresting.com/?p=473 due to a “genetic glitch” now at some point the ability to distinguish extra colours may prove life saving allowing for natural selection to work saving the people who can see more.

although obviously inbreeding will eventually cause severe mutations and not evolution a instance of it every 20 or so generations may cause a degree of evolution that will prove beneficial
(please note that I don’t personally approve of inbreeding I just simply see a hidden possible advantage to limited mutations with in the genetic code.)

(note also for the religious people who dislike the idea of evolution I don’t want to start a religious debate but if god in his infinite wisdom can create complex life maybe he started small and steered in to help Adam and eve when they evolved.)

sulkykid

Posted 13 October 2006 at 07:48 am

psyOtic said: ” … a disturbing point to look at with inbreeding we look at it in current times as disgusting and absurd how ever we all at some point in our family tree are the result of inbreeding… (of course thats only if you believe in evolution.) … “

Actually, Biblical creationists would also believe that we are all creatures of inbreeding as well. Are there any set of beliefs that would not?

mmmpopcorn

Posted 13 October 2006 at 10:08 am

sulkykid said: “Actually, Biblical creationists would also believe that we are all creatures of inbreeding as well. Are there any set of beliefs that would not?”

Religious arguments really aren’t necessary here, as I believe researching any individual’s genealogy back more than a few generations would reveal a couple of relatives marrying each other (Four generations back for myself.) For much of human history, inbreeding was pretty much impossible to escape, and in some cases, expected (think of the royal lines of medieval Europe or ancient Egypt.) In some isolated places, it still is. If you think about it, there’s little reason for it to be disgusting except for the cultural taboos we’ve placed on it and the occasional bizarre genetic disorder that crops up.

Tink

Posted 13 October 2006 at 08:32 pm

Researcher said: “All the bickering is interesting but the article by Saint-Hilaire MH, Saint-Hilaire JM, and Granger L. is correct. The problem they had is that they could not name the source of stimulus to cause operant conditioning. That source was visual Subliminal Distraction. .”

Thanks Researcher!

Alan you did it again, DI! I’m tickled that while reading the article that both first thoughts I came up with (Fainting goats and Tourettes) was addressed before the end, LOL.

As to you all fighting over inbreeding…Please, if your religious then who do you think Cain, Able and Seth married if not their sisters. Surely not their mother Eve(Gasp!).
If your a proponent of evolutionary thinking well then I suppose the options would be a bit wider. Any way it seems to be a moot point with Researchers clear explanation.

Love this DI site! Hey, and I’ve started to develope this strange compulsion to eat pie when ever I log on here…whats up with that? LOL ;)

Julie

Posted 13 October 2006 at 09:36 pm

Berkana said: “Incidentally, there is a family of inbred Turkish Kurds who walk on all fours. They are all mentally retarded, and it seems that their grandparents are very closely related.

Who knew inbreeding could be such an endless source of entertaining disorders?”

Secret Ninja said: “AHAHAHAHAHA”

Honestly, people, where is your compassion?

PresMatt

Posted 14 October 2006 at 12:41 am

Just as interesting as it was the first time I read it… =/

needles

Posted 15 October 2006 at 04:52 pm

I found this site on consanguinity (cousin marriage) that says inbreeding is really common.
“It is estimated that 20 percent of all couples worldwide are first cousins. It is also estimated that 80 percent of all marriages historically have been between first cousins!”http://www.cousincouples.com/info/facts.shtml
I seriously doubt that. Also the site does not cite any sources or researches.

sierra_club_sux

Posted 15 October 2006 at 10:10 pm

Julie said: “Honestly, people, where is your compassion?”

Must be southern Cal… no humor there…

NinerSevenTango

Posted 23 October 2006 at 07:07 am

VIRUS ALERT:

AVG Antivirus reports that following the link to Researcher’s site results in an attempted installation of a backdoor trojan that exploits a windows server service vulnerability.

Tries to install a file called Win1.exe.

If you’ve been there, scan your system!

–97T–

cowenowner

Posted 09 November 2006 at 06:49 am

If the disease is caused primarily by a mutation of the glycine receptor it makes for some interesting conclusions about how the brain works. Glycine is an inhibitory neurotransmitter and concentrated in the spinal cord. If these patients are following orders or echoing commands it would suggest that the capacity to inhibit these habitual stimulus-response pairs occurs at the level of the spinal cord. To me that is surprising, especially for verbal commands which must take some degree of cognitive processing, as I usally think of such inhibition as occuring in the brain and the motor command being blocked at the brain level. Our friends the Jumping Frenchment seem to suggest that all of our repressed responses make it all the way down to the spinal cord before they are repressed. Cool. Viva the lizard brain.

I guess that means if I want to know everyones hidden desires (well responses at least), all I have to do is implant some electrodes into the spinal cord. Any volunteers?

-C

tom_squick

Posted 18 January 2007 at 10:23 am

I think I work with one of these guys. He has like 3 rear view mirrors on his monitor and no matter how gently I announce myself, he just about jumps out of his skin.

adamj.

Posted 08 February 2007 at 06:26 pm

41st!

Ok, first things first, again what a great site Di is!

Fredrated said: “”evolution describes the fact that in a given set of organisms, those which manage to survive are the same ones that do most of the breeding.”

Do we know how much of the breeding is done by those that don’t survive?”

HaHaHaHaHa!

ifeelya

Posted 06 March 2007 at 03:14 am

Oh, sweet jayzus. Would you people pu-leeze let go of the psychobabble? Any neurotoxin will do some wild stuff to the nervous system. Check out excitotoxins. Fascinating little things. I may just have to post an article on what it’s like to live with that kind of damage. And then I may have to post another on what it’s like to deal with Psych majors who don’t have to live with it. Did you know that some people with that kind of startle reflex experience it as a kind of whole-body nerve pain?

Then you end up guilt-tripping dunderheads who enjoy torturing you because you’d have to wire AC current to their testicles to really get even. And that would be illegal. Well, unless they’re into that sort of thing. Or unless they’re Psych majors, I guess. Then it’s a damn interesting experiment and they should get extra credit.

vacuumcleanerbot

Posted 01 June 2007 at 04:58 pm

I’m reminded of Cosmo Kramer.

lizdini

Posted 12 March 2008 at 08:00 pm

This has gotten really off point from the article, but heres my two cents:

Almost all cultures have some taboo on incest, most likely as a way to prevent unwanted mutations, some more then others; like being able to marry your first cousin but not your uncle, or your mother’s sister’s children but not her brother’s children.

Although I have no direct incest in my line as far as I’m aware (although it’s true that mathamaticly it’s impossible not to at some point in the past), my Grandfather’s Aunt is also his Grandmother. He is desended from her step-father. And before you judge, you try being a single woman past marrying prime in the 1920’s.

I agree that saying someone’s afliction is amusing is both childish and perverse, regardless of the reason for the afliction. You certainly wouldn’t care for it if the situation was reversed.

Rodger Wrighthead

Posted 26 August 2009 at 02:54 am

I remember reading that incest results in no mutations in offspring but a higher likelihood of the full expression of recessive genes which could result in defects in the offspring (a big easy example being sickle cell anemia). As far as “unwanted mutations” go you are not more likely to spontaniously mutate if your parents happen to be related. That doesn’t however make it any less icky…

Misty

Posted 31 October 2015 at 02:40 am

Thank you for this article. My nephew has many health problems. They are testing for abnormal genes. This article helps to understand this illness. Thank you

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